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JOURNAL ARTICLE
Michael J Diaz, Mahtab Forouzandeh, Tyler Werbel, Kiran Motaparthi
No abstract text is available yet for this article.
May 2024: JAAD Case Reports
#2
JOURNAL ARTICLE
Daria V Babushok, Amy E DeZern, Carlos de Castro, Zora R Rogers, David Beenhouwer, Michael S Broder, Suzanne Fanning, Sarah N Gibbs, Rabi Hanna, Jaroslaw P Maciejewski, Bart L Scott, Srinivas K Tantravahi, Marcin W Wlodarski, Irina Yermilov, Bhumika J Patel
Severe aplastic anemia (SAA) is a rare hematologic condition for which there is no clear management algorithm. A panel of 11 adult and pediatric experts on aplastic anemia was assembled and, using the RAND/UCLA modified Delphi panel method, evaluated >600 varying patient care scenarios to develop clinical recommendations for the initial and subsequent management of patients of all ages with SAA. Here we present the panel's recommendations to rule out inherited bone marrow failure (IBMF) syndromes, on supportive care prior to and during first-line therapy, and on first-line (initial management) and second-line (subsequent management) therapy of acquired SAA, focusing on when transplant versus medical therapy is most appropriate...
April 26, 2024: Blood Advances
#3
REVIEW
Matteo Riccò, Salvatore Parisi, Silvia Corrado, Federico Marchesi, Marco Bottazzoli, Davide Gori
Human Respiratory Syncytial Virus (RSV) is a common cause of respiratory tract infections. Usually associated with infants and children, an increasing amount of evidence suggests that RSV can cause substantial morbidity and mortality in immunocompromised individuals, including recipients of bone marrow transplantation (BMT). The present systematic review was therefore designed in accordance with the PRISMA guidelines to collect available evidence about RSV infections in BMT recipients. Three medical databases (PubMed, Embase, and MedRxiv) were therefore searched for eligible observational studies published up to 30 September 2023 and collected cases were pooled in a random-effects model...
March 29, 2024: Infectious Disease Reports
#4
JOURNAL ARTICLE
Ceyhun Bozkurt, Volkan Hazar, Barış Malbora, Alphan Küpesiz, Utku Aygüneş, Tunç Fışgın, Musa Karakükçü, Barış Kuşkonmaz, Suar Çakı Kılıç, Derya Bayırlı, Özlem Arman Bilir, Koray Yalçın, Salih Gözmen, Vedat Uygun, Murat Elli, Hakan Sarbay, Funda Tayfun Küpesiz, Hatice İlgen Şaşmaz, Başak Adaklı Aksoy, Ebru Yılmaz, Fatma Visal Okur, Funda Tekkeşin, Fatma Demir Yenigürbüz, Gülcihan Özek, Abdullah Avni Atay, İkbal Ok Bozkaya, Suna Çelen, Seda Öztürkmen, Adalet Meral Güneş, Orhan Gürsel, Elif Güler, Alper Özcan, Duygu Uçkan Çetinkaya, Selime Aydoğdu, Namık Yaşar Özbek, Gülsün Karasu, Gülay Sezgin, Ömer Doğru, Davut Albayrak, Gülyüz Öztürk, Serap Aksoylar, Hayriye Daloğlu, Işık Odaman Al, Melike Sezgin Evim, Sinan Akbayram, Yurday Öncül, Emine Zengin, Canan Albayrak, Çetin Timur, Yeter Düzenli Kar, Hasan Fatih Çakmaklı, Özlem Tüfekçi, Ersin Töret, Bülent Antmen
BACKGROUND: Data on the risk factors and outcomes for pediatric patients with SARS-CoV-2 infection (COVID-19) following hematopoietic stem cell transplantation (HSCT) are limited. OBJECTIVES: The study aimed to analyze the clinical signs, risk factors, and outcomes for ICU admission and mortality in a large pediatric cohort who underwent allogeneic HSCT prior to COVID-19 infection. METHOD: In this nationwide study, we retrospectively reviewed the data of 184 pediatric HSCT recipients who had COVID-19 between March 2020 and August 2022...
May 2024: Pediatric Transplantation
#5
JOURNAL ARTICLE
Claire Freycon, Edith Sepulchre, Vincent-Philippe Lavallée, David Mitchell, Margaret L MacMillan, Catherine Vezina, Catherine Goudie
Acute promyelocytic leukemia (APL) represents 5%-10% of childhood acute myeloid leukemia (AML) and is the most curable subtype of AML. Fanconi anemia (FA) is one of the most common inherited bone marrow failure syndromes caused by biallelic pathogenic variants (PV) in specific DNA-repair genes. Biallelic PVs in FANCD1/BRCA2 (FA-D1) account for 3% of FA and are associated with early-onset leukemia and a high risk of solid tumors. We report a 4 year-old boy from non-consanguineous parents diagnosed with standard risk APL...
April 24, 2024: Clinical Genetics
#6
JOURNAL ARTICLE
Gökcan Öztürk, Deniz Bayrakoğlu, Şule Haskoloğlu, Kübra Baskın, Nazlı Deveci, Elif İnce, Talia İleri, Hasan Çakmaklı, Mehmet Ertem, Aydan İkincioğulları, Figen Doğu
BACKGROUND/AIM: There are several complications of hematopoietic stem cell transplantation. Without any doubt, most important of these is aGvHD that increases transplant-related mortality. The aim of this study is to investigate whether ST-2 and Reg3α levels measured at an early stage in pediatric patients undergoing allogeneic hematopoietic stem cell transplantation can be individual biomarkers identifying future GvHD and predicting treatment response. MATERIALS AND METHODS: From January 2019 to January 2021, 27 patients undergoing hematopoietic stem cell transplantation for primary immunodeficiency or hematopoietic diseases formed the study group...
April 17, 2024: Hematology, Transfusion and Cell Therapy
#7
Bibi Shahin Shamsian, Nader Momtazmanesh, Hedyeh Saneifard, Seyed Mohammad Taghi Hosseini Tabatabaei, Mohammadreza Jafari, Zahra Khafaf Pour, Kawthar Jasim Mohammad Rida Al-Hussieni, Mahnaz Jamee, Sharareh Kamfar
BACKGROUND: Osteopetrosis is a group of geneticall heterogeneous disorders resulting from impaired osteoclast function and bone resorption. The identification of specific genetic mutations can yield important prognostic and therapeutic implications. Herein, we present the diagnosis and successful application of hematopoietic stem cell transplantation (HSCT) in a patient with osteopetrosis caused by carbonic anhydrase II deficiency (Intermediate osteopetrosis). CASE PRESENTATION: Herein, we describe a 2...
May 2024: Pediatric Transplantation
#8
JOURNAL ARTICLE
Vasil Toskov, Annamaria Cseh, Alexander Claviez, Beatrice Drexler, Natalia Rotari, Stephan Schwarz-Furlan, Matthias Braun, Peter Bader, Peter Lang, Rita Beier, Bernhard Erdlenbruch, Monika Führer, Miriam Erlacher, Charlotte M Niemeyer, Brigitte Strahm, Ayami Yoshimi
We report 5 children with bone marrow failure (BMF) after primary varicella zoster virus (VZV) infection or VZV vaccination, highlighting the highly variable course. Two patients were treated with intravenous immunoglobulins; one had a slow hematologic recovery, and the other was rescued by allogeneic hematopoietic stem cell transplantation (HSCT). Of the 2 patients treated with immunosuppressive therapy with antithymocyte globulin and cyclosporine, one had a complete response, and the other was transplanted for nonresponse...
April 23, 2024: Journal of Pediatric Hematology/oncology
#9
Meenakshi Gopalakrishnan, Arunalini Ramanathan, Dhaarani Jayaraman, Sri Gayathri Shanmugam, Julius Xavier Scott
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hematological disorder of immune dysregulation associated with significant challenges in diagnosis and management. Described as primary HLH secondary to genetic defects or more commonly secondary to infections, it can also occur secondary to malignancy, i.e., malignancy-associated hemophagocytic lymphohistiocytosis (M-HLH). A five-year-old male child presented with left cervical adenopathy and a high-spiking fever for two weeks. He had pallor, anasarca, multiple enlarged and matted cervical lymph nodes, respiratory distress, and hepatomegaly...
March 2024: Curēus
#10
REVIEW
Agnieszka Piekarska, Katarzyna Pawelec, Anna Szmigielska-Kapłon, Marek Ussowicz
Acquired aplastic anemia (AA) is an immune-mediated bone marrow (BM) failure where marrow disruption is driven by a cytotoxic T-cell-mediated autoimmune attack against hematopoietic stem cells. The key diagnostic challenge in children, but also in adults, is to exclude the possible underlying congenital condition and myelodysplasia. The choice of treatment options, either allogeneic hematopoietic cell transplantation (alloHCT) or immunosuppressive therapy (IST), depends on the patient's age, comorbidities, and access to a suitable donor and effective therapeutic agents...
2024: Frontiers in Immunology
#11
JOURNAL ARTICLE
Nancy A Kernan, Elizabeth Klein, Audrey Mauguen, Joanne Torok-Castanza, Susan E Prockop, Andromachi Scaradavou, Kevin Curran, Barbara Spitzer, Maria Cancio, Julie Ruggiero, Jennifer Allen, Andrew Harris, Joseph Oved, Richard J O'Reilly, Jaap Jan Boelens
BACKGROUND: The clinical value of serial routine bone marrow aspirates (rBMAs) in the first year after allogeneic hematopoietic cell transplantation (alloHCT) to detect or predict relapse of acute leukemia (AL) and myelodysplastic syndrome (MDS) in pediatric and young adult patients is unclear. OBJECTIVE: The purpose of this analysis was to determine if assessment of minimal residual disease (MRD) by multiparameter flow cytometry (MFC, MFC-MRD) or donor chimerism (DC) in rBMAs or serial CBCs done in the year after alloHCT predicted relapse of AL or MDS in pediatric and young adult patients...
April 20, 2024: Transplantation and cellular therapy
#12
JOURNAL ARTICLE
Gabriel Salinas Cisneros, Christopher C Dvorak, Janel Long-Boyle, Sandhya Kharbanda, Kristin A Shimano, Alexis Melton, Julia Chu, Lena E Winestone, Jasmeen Dara, James N Huang, Michelle L Hermiston, Matt Zinter, Christine S Higham
INTRODUCTION: Sinusoidal obstructive syndrome (SOS), or veno-occlusive disease (VOD), of the liver has been recognized as a complex, life-threatening complication in the post-hematopoietic stem cell transplant (HSCT) setting. The diagnostic criteria for SOS have evolved over the last several decades with a greater understanding of the underlying pathophysiology, with two recent diagnostic criteria introduced in 2018 (EBMT criteria) and 2020 (Cairo criteria). OBJECTIVE: We sought out to evaluate the performance characteristics in diagnosing and grading SOS in pediatric patients of the four different diagnostic criteria (Baltimore, Modified Seattle, EBMT, and Cairo) and severity grading systems (defined by the EBMT and Cairo criteria)...
April 15, 2024: Transplantation and cellular therapy
#13
JOURNAL ARTICLE
Y Z Zhao, H H Ma, H Y Lian, D Wang, T Y Wang, R Zhang
Objective: To analyze the efficacy and safety of the L-DEP regimen (asparaginase, liposome doxorubicin, etoposide and methylprednisolone) as a salvage therapy for the refractory primary hemophagocytic lymphohistocytosis triggered by Epstein-Barr virus infection (EBV-pHLH) in children. Methods: In this retrospective case study, clinical and laboratory data before and after L-DEP regimen of 4 children diagnosed with EBV-pHLH in Beijing Children's hospital between January 2016 and June 2022 were collected, and the efficacy and safety of L-DEP regimen for the treatment of EBV-pHLH were analyzed...
April 16, 2024: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
#14
JOURNAL ARTICLE
Jowita Frączkiewicz, Katarzyna Pawińska-Wąsikowska, Katarzyna Szymbor, Walentyna Balwierz, Szymon Skoczeń, Krzysztof Czyżewski, Sylwia Kołtan, Jan Styczyński, Anna Małecka, Ninela Irga-Jaworska, Joanna Trelińska, Wojciech Młynarski, Olga Zając-Spychała, Agnieszka Sobkowiak-Sobierajska, Katarzyna Derwich, Wioletta Bal, Radosław Chaber, Agnieszka Książek, Tomasz Szczepański, Joanna Zawitkowska, Katarzyna Drabko, Agnieszka Chodała-Grzywacz, Grażyna Karolczyk, Christopher Kobierzycki, Krzysztof Kałwak
Background: Patients treated with hemato-oncological malignancies (HO) or undergoing cellular therapies such as hematopoietic stem cell transplantation (HSCT) or chimeric antigen receptor T cells (CAR-T) were significantly affected by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Despite the success of SARS-CoV-2 vaccination, immunocompromised patients remain at increased risk for severe coronavirus disease (COVID-19), rendering this group of population a high priority for additional prevention and treatment options...
March 31, 2024: Journal of Clinical Medicine
#15
JOURNAL ARTICLE
Bo Kyung Kim, Kyung Taek Hong, Jung Yoon Choi, Hyery Kim, Hyun Jin Park, Hyoung Jin Kang
Traditionally, bone marrow (BM) has been preferred as a source of stem cells (SCs) in pediatric hematopoietic SC transplantation (HSCT); however, the use of peripheral blood SCs (PBSC) has recently increased. With advancing graft-versus-host disease (GVHD) prophylaxis, whether the BM is still a better SC source than PB in sibling donor HSCT remains controversial. Here, we compared the results of BM transplantation (BMT) and PBSC transplantation (PBSCT) in pediatric patients with malignant or non-malignant diseases receiving sibling HSCT using a total of 7...
April 10, 2024: Annals of Hematology
#16
JOURNAL ARTICLE
Deema Gashgarey, Mohammed Alsuhaibani, Raghad Alhuthil, Hattan Alhabshan, Azzam Alabdulqader, Rakan Badran, Abdulaziz Balhmar, Haifa Aldawood, Esam A AlBanyan, Salem AlGhamdi, Suliman AlJumaah, Ohoud AlYabes, Sami Al-Hajjar
BACKGROUND: The burden of respiratory syncytial virus (RSV) in high-risk pediatric patients remains unclear. Therefore, this study aims to characterize pediatric RSV cases from January 2019 to December 2022 and assess the impact of the COVID-19 pandemic on RSV burden and RSV-related outcomes. In addition, examining factors influencing RSV-related hospitalization. METHODS: This is a retrospective study that included pediatric patients (aged 14 and below) who presented at King Faisal Specialist Hospital and Research Centre (KFSHRC) in Riyadh, Saudi Arabia with RSV infection identified using real-time reverse-transcriptase polymerase chain reaction assays...
April 4, 2024: Journal of Epidemiology and Global Health
#17
REVIEW
Maulidina Agustin, Anita Mahadewi, Retno Danarti
Epidermolysis bullosa (EB) is a genodermatosis that lacks effective treatments and requires supportive care for its severe, life-threatening manifestations. Bone marrow transplantation (BMT) and its derived cells have been suggested to improve clinical symptoms and quality of life. A comprehensive search was conducted for publications evaluating BMT and bone marrow-derived mesenchymal stem cell (BM-MSC) therapy for EB in PubMed/MEDLINE, Google Scholar, and Cochrane databases from inception until June 2023. A total of 55 participants with severe forms of EB had BMT and/or BM-MSCs, with recessive dystrophic EB as the most common EB type; 53 (96...
April 1, 2024: Pediatric Dermatology
#18
Vraj Bhatt, Sunidhi Rohatgi, Mansi Singh
KEY CLINICAL MESSAGE: Fanconi anemia with Mitomycin C sensitivity is a rare, complex hematological condition. Our case study emphasizes the significance of early diagnosis, appropriate genetic testing, and cautious use of chemotherapeutic agents. ABSTRACT: Fanconi anemia (FA) is a rare genetic disorder characterized by bone marrow failure, congenital anomalies, and predisposition to cancer. Here, we present the case of a 6-year-old boy with a known diagnosis of Fanconi anemia who exhibited sensitivity to Mitomycin C...
April 2024: Clinical Case Reports
#19
JOURNAL ARTICLE
Allison L Bartlett, John E Wagner, Blaise V Jones, Susanne Wells, Anthony Sabulski, Christine Fuller, Stella M Davies
Fanconi anemia (FA) is a complex inherited bone marrow failure syndrome characterized by chromosomal instability and defective DNA repair causing sensitivity to DNA interstrand cross-linking agents. Our understanding of the full adult phenotype of the disease continues to evolve, as most patients with Fanconi Anemia died of marrow failure in the first decade of life prior to more recent advances in allogeneic hematopoietic cell transplantation. Herein, we report a previously undescribed, clinically concerning, progressive neurologic syndrome in patients with FA...
March 24, 2024: Blood Advances
#20
JOURNAL ARTICLE
Stefano Giardino, Dirk-Jan Eikema, Brian Piepenbroek, Mattia Algeri, Mouhab Ayas, Maura Faraci, Abdelghani Tbakhi, Marco Zecca, Mohammed Essa, Bénédicte Neven, Yves Bertrand, Gaurav Kharya, Tatiana Bykova, Sarah Lawson, Mario Petrini, Alexander Mohseny, Fanny Rialland, Beki James, Anca Colita, Mony Fahd, Simone Cesaro, Ansgar Schulz, Katharina Kleinschmidt, Krzysztof Kałwak, Selim Corbacioglu, Carlo Dufour, Antonio Risitano, Régis Peffault de Latour
Haploidentical stem cell transplantation (haplo-SCT) represents the main alternative for children with inherited bone marrow failure syndrome (I-BMF) lacking a matched donor. This retrospective study, conducted on behalf of the EBMT SAAWP and PDWP, aims to report the current outcomes of haplo-SCT in I-BMFs, comparing the different in vivo and ex vivo T-cell depletion approaches. One hundred and sixty-two I-BMF patients who underwent haplo-SCT (median age 7.4 years) have been registered. Fanconi Anemia was the most represented diagnosis (70...
March 18, 2024: American Journal of Hematology
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